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Adrenocortical Carcinoma: Clinical Features & Hormonal Evaluation

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This slide contains general information about Adrenocortical Carcinoma, how patients presents with it and information regarding how it can be Hormonally diagnosed.

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ADRENOCORTICAL CARCINOMA PRESENTATION & HORMONAL EVALUATION Gourav Singh Dept. of Endocrinology AIIMS 1
INTRODUCTION & EPIDEMIOLOGY ACC is a rare malignancy with an annual incidence of 12 per million population. Around 5% of all Adrenal Incidentaloma. Highly malignant tumor. Bimodal age distribution Children First decade of life Adults Fourth to Fifth decades of life F > M (55-60% more in females). 2
INTRODUCTION & EPIDEMIOLOGY ACC usually present as sporadic tumor. But it can present as Hereditary Tumor Associated with Germline TP53 mutation (Li-Fraumeni syndrome) Alterations in the Wnt/硫-catenin pathway (FAP) IGF2 overexpression (Beckwith Wiedemann Syndrome) 3
CLINICAL FEATURES Detected incidentally on CT. 6070% of ACCs show biochemical evidence of steroid overproduction Cortisol (Most Common) Aldosterone Sex Steroids Mixed excess production of several corticosteroids is indicative of malignancy. So may present as Functional/Non- Functional tumors 4
CLINICAL FEATURES Non-Functional tumor symptoms Local symptoms: Abdominal fullness, back pain, nausea & vomiting. Systemic/Metastatic symptoms: Anorexia, anaemia, asthenia and weight loss. 5
CLINICAL FEATURES Functional tumor symptoms Symptoms like Cushing Syndrome (Cortisol secreting tumor). 6
CLINICAL FEATURES 7
CLINICAL FEATURES Functional tumor symptoms Symptoms like Cushing Syndrome (Cortisol secreting tumor). Symptoms like Hyperaldosteronism. 8
CLINICAL FEATURES 9
HORMONAL EVALUATION 10
HORMONAL EVALUATION Dexamethasone suppression test To confirm the diagnosis of Cushing Syndrome. 11
HORMONAL EVALUATION 12
HORMONAL EVALUATION Dexamethasone suppression test To confirm the diagnosis of Cushing Syndrome. Dexamethasone given in night (11 pm) Cortisol levels measured on next morning (8 am) Done in two stages Low dose test 1mg Dexamethasone given To confirm patient actually has Cushing Syndrome. Cortisol > 5gm/dl High dose test 8mg Dexamethasone given To know the cause of Cushing Syndrome. 13
11p.m. 11p.m. 14
REFERENCES AND ILLUSTRATIONS Harrison's Principles of Internal Medicine, 20th edition. Puglisi S, Perotti P, Cosentini D, Roca E, Basile V, Berruti A, Terzolo M. Decision- making for adrenocortical carcinoma: surgical, systemic, and endocrine management options. Expert review of anticancer therapy. 2018 Nov 2;18(11):1125-33. Ross NS, Aron DC. Hormonal evaluation of the patient with an incidentally discovered adrenal mass. New England Journal of Medicine. 1990 Nov 15;323(20):1401-5. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. The Journal of urology. 2003 Jan;169(1):5-11. Google Image Search. 15

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Adrenocortical Carcinoma: Clinical Features & Hormonal Evaluation

  • 1. ADRENOCORTICAL CARCINOMA PRESENTATION & HORMONAL EVALUATION Gourav Singh Dept. of Endocrinology AIIMS 1
  • 2. INTRODUCTION & EPIDEMIOLOGY ACC is a rare malignancy with an annual incidence of 12 per million population. Around 5% of all Adrenal Incidentaloma. Highly malignant tumor. Bimodal age distribution Children First decade of life Adults Fourth to Fifth decades of life F > M (55-60% more in females). 2
  • 3. INTRODUCTION & EPIDEMIOLOGY ACC usually present as sporadic tumor. But it can present as Hereditary Tumor Associated with Germline TP53 mutation (Li-Fraumeni syndrome) Alterations in the Wnt/硫-catenin pathway (FAP) IGF2 overexpression (Beckwith Wiedemann Syndrome) 3
  • 4. CLINICAL FEATURES Detected incidentally on CT. 6070% of ACCs show biochemical evidence of steroid overproduction Cortisol (Most Common) Aldosterone Sex Steroids Mixed excess production of several corticosteroids is indicative of malignancy. So may present as Functional/Non- Functional tumors 4
  • 5. CLINICAL FEATURES Non-Functional tumor symptoms Local symptoms: Abdominal fullness, back pain, nausea & vomiting. Systemic/Metastatic symptoms: Anorexia, anaemia, asthenia and weight loss. 5
  • 6. CLINICAL FEATURES Functional tumor symptoms Symptoms like Cushing Syndrome (Cortisol secreting tumor). 6
  • 8. CLINICAL FEATURES Functional tumor symptoms Symptoms like Cushing Syndrome (Cortisol secreting tumor). Symptoms like Hyperaldosteronism. 8
  • 11. HORMONAL EVALUATION Dexamethasone suppression test To confirm the diagnosis of Cushing Syndrome. 11
  • 13. HORMONAL EVALUATION Dexamethasone suppression test To confirm the diagnosis of Cushing Syndrome. Dexamethasone given in night (11 pm) Cortisol levels measured on next morning (8 am) Done in two stages Low dose test 1mg Dexamethasone given To confirm patient actually has Cushing Syndrome. Cortisol > 5gm/dl High dose test 8mg Dexamethasone given To know the cause of Cushing Syndrome. 13
  • 15. REFERENCES AND ILLUSTRATIONS Harrison's Principles of Internal Medicine, 20th edition. Puglisi S, Perotti P, Cosentini D, Roca E, Basile V, Berruti A, Terzolo M. Decision- making for adrenocortical carcinoma: surgical, systemic, and endocrine management options. Expert review of anticancer therapy. 2018 Nov 2;18(11):1125-33. Ross NS, Aron DC. Hormonal evaluation of the patient with an incidentally discovered adrenal mass. New England Journal of Medicine. 1990 Nov 15;323(20):1401-5. Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. The Journal of urology. 2003 Jan;169(1):5-11. Google Image Search. 15